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Startle and its disorders.
Neurophysiol Clin. 2006 Sep-Dec; 36(5-6):357-64.NC

Abstract

Exaggerated startle is an uncommon feature of various neurological diseases, but is still lacking precise analysis in many of them. So far, electrophysiologic and cinematographic analyses allow discriminating two main subtypes. The prototype of primary exaggerated startle is hereditary hyperekplexia, a well-studied disorder of the inhibitory glycine receptor and thus of the neuronal Cl- channel. The involuntary jerking in hereditary hyperekplexia is considered a reticular reflex myoclonus. The prototype of primary normal startle with secondary abnormalities is startle epilepsy where a surprise stimulus typically provokes a normal startle, which in turn initiates a focal (most often frontal lobe) seizure with tonic posturing of the limbs. Clinical differential diagnosis between both subtypes may be difficult in individual cases, but there are abnormalities in clinical and neurophysiologic reflex testing, which need, however, broad validation.

Authors+Show Affiliations

Neurologische Universitätsklinik, Im Neuenheimerfeld 400, D 69120 Heidelberg, Germany. hans-michael_meinck@med.uni-heidelberg.de

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

17336782

Citation

Meinck, H-M. "Startle and Its Disorders." Neurophysiologie Clinique = Clinical Neurophysiology, vol. 36, no. 5-6, 2006, pp. 357-64.
Meinck HM. Startle and its disorders. Neurophysiol Clin. 2006;36(5-6):357-64.
Meinck, H. M. (2006). Startle and its disorders. Neurophysiologie Clinique = Clinical Neurophysiology, 36(5-6), 357-64.
Meinck HM. Startle and Its Disorders. Neurophysiol Clin. 2006 Sep-Dec;36(5-6):357-64. PubMed PMID: 17336782.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Startle and its disorders. A1 - Meinck,H-M, Y1 - 2007/01/26/ PY - 2007/3/6/pubmed PY - 2007/7/3/medline PY - 2007/3/6/entrez SP - 357 EP - 64 JF - Neurophysiologie clinique = Clinical neurophysiology JO - Neurophysiol Clin VL - 36 IS - 5-6 N2 - Exaggerated startle is an uncommon feature of various neurological diseases, but is still lacking precise analysis in many of them. So far, electrophysiologic and cinematographic analyses allow discriminating two main subtypes. The prototype of primary exaggerated startle is hereditary hyperekplexia, a well-studied disorder of the inhibitory glycine receptor and thus of the neuronal Cl- channel. The involuntary jerking in hereditary hyperekplexia is considered a reticular reflex myoclonus. The prototype of primary normal startle with secondary abnormalities is startle epilepsy where a surprise stimulus typically provokes a normal startle, which in turn initiates a focal (most often frontal lobe) seizure with tonic posturing of the limbs. Clinical differential diagnosis between both subtypes may be difficult in individual cases, but there are abnormalities in clinical and neurophysiologic reflex testing, which need, however, broad validation. SN - 0987-7053 UR - https://www.unboundmedicine.com/medline/citation/17336782/Startle_and_its_disorders_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0987-7053(06)00136-5 DB - PRIME DP - Unbound Medicine ER -